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目的评价靶向扩张肺血管药物治疗Fontan儿童患者的疗效性及安全性。方法回顾性分析2013年1~12月共42例在阜外心血管病医院行Fontan手术患者的基本资料,包括术前导管及超声资料,术前、术中及术后血流动力学资料,血标本资料及术后应用肺血管扩张药物情况。术后除常规治疗外,根据应用靶向扩张肺血管药物将患者分为用药组24例及对照组18例。结果 42例患者住院期间无死亡。术后单独应用波生坦6例,单独应用西地那非7例,联合波生坦和西地那非治疗者11例。用药组手术年龄较对照组低(P=0.082),术后需开窗比例用药组较对照组高(P=0.120),但差异均无统计学意义;用药组术后1 d乳酸明显高于对照组,差异有统计学意义(P=0.031)。4例术中主动脉阻断患者术后均应用了降肺动脉高压药物(P=0.122)。呼吸机使用时间用药组明显较对照组长(P=0.050)。用药组住院时间、住ICU时间及胸腔引流时间均较对照组长,但差异无统计学意义。出院时平均脉搏血氧饱和度用药组较对照组明显降低(P=0.065)。24例应用靶向扩张肺血管药物的Fontan患儿对比用药前后无肝、肾功能损害发生。结论靶向肺血管扩张药物多用于重症Fontan术后患者,如年龄小、手术过程复杂、术后心功能差、中心静脉压高、开窗等。本研究未能证实靶向肺血管扩张药物能帮助改善患者预后,缩短住院时间、住ICU时间及胸腔引流时间。
Objective To evaluate the efficacy and safety of targeted extended pulmonary vascular drug treatment in children with Fontan. Methods A retrospective analysis of 42 patients with Fontan operation in Fu Wai Hospital from January 2013 to December 2013 was performed. The data including preoperative catheter and ultrasound data, preoperative, intraoperative and postoperative hemodynamic data, Blood samples and postoperative pulmonary vasodilator drugs. In addition to conventional treatment, patients were divided into medication group (n = 24) and control group (n = 18) according to the application of targeted pulmonary vasodilator drugs. Results 42 patients died during hospitalization. Postoperative application of bosentan alone in 6 cases, alone, 7 cases of sildenafil, combined bosentan and sildenafil treatment in 11 cases. The operative age in the medication group was lower than that in the control group (P = 0.082), and the proportion of patients who needed to open the window after operation was higher than that in the control group (P = 0.120), but the difference was not statistically significant. The control group, the difference was statistically significant (P = 0.031). Four patients undergoing aortic occlusion were treated with pulmonary hypertension drug (P = 0.122). Ventilator use time was significantly longer than the control group (P = 0.050). The hospitalization time, ICU time and chest drainage time were longer than the control group, but the difference was not statistically significant. Mean discharge pulse oximetry medication group was significantly lower than the control group (P = 0.065). Twenty-four patients with Fontan who were targeted for dilated pulmonary vascular disease developed no liver or kidney dysfunction before and after treatment. Conclusions Targeted pulmonary vasodilator drugs are mostly used in patients with severe Fontan, such as small age, complicated operation process, poor postoperative cardiac function, high central venous pressure and fenestration. This study failed to demonstrate that targeted vasodilators could help improve patient outcomes, reduce length of hospital stay, duration of ICU placement, and time to chest drainage.